Progressive myelin oligodendrocyte glycoprotein-associated demyelination mimicking leukodystrophy

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may be associated with relapsing disease, but clinical progression independent of relapse activity is rare. Objectives: To report progressive disease in a patient with MOGAD. Methods: A single retrospective case report. Results: At 4 years of age, the patient had a single episode of acute disseminated encephalomyelitis. She remained well until age 17 years but over the next 9 years developed progressive spastic quadriparesis, cognitive and bulbar dysfunction. Brain imaging showed a leukodystrophy-like pattern of white matter abnormality with contrast enhancement at different time points. Myelin oligodendrocyte glycoprotein (MOG)-IgG was repeatedly positive by live cell-based assay. Conclusion: Secondary progression may be a rare presentation of MOG-IgG-associated disease.